Lutembacher Syndrome Treatment & Management

Updated: Dec 15, 2020
  • Author: Kamran Riaz, MD; Chief Editor: Yasmine S Ali, MD, MSCI, FACC, FACP  more...
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Medical Care

Diuretics may be used for symptomatic relief of right-sided heart failure. 

Digoxin, beta blockers, and calcium channel blockers are used mainly for rate control of atrial arrhythmias, whereas amiodarone and sotalol are used not only for rate control but also for conversion into and maintenance of normal sinus rhythm.

Patients with Lutembacher syndrome who have undergone complete repair with prosthetic material or a device need subacute bacterial endocarditis (SBE) prophylaxis for the first 6 months after the repair procedure. In addition, patients with previous history of endocarditis warrant SBE prophylaxis.

Diet and activity

Patients should adhere to a low-sodium diet. Activity should be as tolerated by the patient.


Consultation with a cardiothoracic surgeon or interventional cardiologist may be indicated.


Surgical Care

Until relatively recently, surgery was the only definite curative treatment available for Lutembacher syndrome and involved closure of the atrial septal defect (ASD) and mitral commissurotomy or mitral valve replacement. [7]

Percutaneous closure of ASD and mitral balloon valvuloplasty [8, 9, 10, 11, 12]

Percutaneous closure of the ASD with a clamshell device and mitral valvuloplasty provides a nonsurgical approach to correct these defects. Although mitral valvuloplasty has been performed for several decades, percutaneous closure of an ASD with a device represents a still-developing technology.

As already described, mitral valvuloplasty alone can be complicated by development of ASD secondary to transseptal puncture performed as a part of the procedure.

More recently, percutaneous transcatheter treatment with Accura balloon valvuloplasty and Amplatzer septal occluder device closure has been used for successful management of an 18 year old with congenital ASD and acquired rheumatic mitral stenosis. [13]

Indications for surgery or percutaneous intervention include the following:

  • ASD with a Qp/Qs ratio of more than 1.5

  • Moderate-to-severe mitral stenosis

  • Any degree of pulmonary hypertension, except individuals with irreversible pulmonary hypertension (Eisenmenger syndrome, see below)

Surgery is now performed early rather than late because the rates of heart failure and cardiac arrhythmia increase with age. Patients with pulmonary hypertension should demonstrate reversibility of pulmonary vascular resistance prior to surgical (or percutaneous) correction of ASD. Patients with pulmonary hypertension and irreversibly increased pulmonary vascular resistance (ie, Eisenmenger physiology) invariably develop progressive right-sided heart failure after ASD closure and die.

The symptoms of Lutembacher syndrome depend upon the size of the atrial septal defect, severity of mitral stenosis, compliance of the right ventricle, and pulmonary artery hypertension. Khanna et al reported on a patient with Lutembacher syndrome who had severe pulmonary hypertension and underwent successful surgical repair. [14]


Long-Term Monitoring

Follow-up care of patients with Lutembacher syndrome includes subacute bacterial endocarditis prophylaxis and surveillance for worsening signs or symptoms of heart failure. Periodic echocardiograms are indicated to assess the severity of mitral stenosis and the magnitude of left-to-right shunt and pulmonary artery pressure.

Periodic assessment of the following:

  • Blood urea nitrogen (BUN) levels

  • Creatinine levels

  • Serum electrolyte levels in patients taking diuretics